Treatment of Stage IV Favorable Histology Wilms Tumor in Children with Metastatic Disease

This protocol applies to children diagnosed with stage IV favorable histology Wilms tumor classified as higher risk, in whom metastatic disease is present — most commonly involving the lungs.

Clinical Scenario

The defining features of this population are pediatric age, stage IV disease, favorable histology, higher-risk classification, and the presence of metastases. Pulmonary metastases are the most frequent site of spread in this setting.

Key Treatment Goal

Complete response of lung-only metastases at the six-week assessment point during chemotherapy.

Treatment Approach

Initial therapy follows the DD4A chemotherapy protocol — a structured multi-agent regimen administered over an extended treatment course. Radiation therapy, including consideration of pulmonary irradiation, is integrated based on disease extent and the response observed at the six-week evaluation.

The complete regimen, radiation criteria, and decision algorithm are available in the full protocol.

References

DD4A is recommended as initial therapy for patients with stage IV FHWT classified as higher risk.

DOI: 10.6004/jnccn.2021.0037

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